ABSTRACT
INTRODUCTION: Among microangiopathic disorders of pregnancy, catastrophic antiphospholipid syndrome (CAPS) is a maternal and fetal life-threatening disorder. Hepatic involvement of this multi-systemic disorder can be confused with HELLP syndrome, occurring usually later in the course of pregnancy. CASE REPORT: We report a case of probable CAPS with hepatic disease in a pregnant woman at 13 week's gestation, with antiphospholipid syndrome and biological features of HELLP syndrome. Unspecific hepatic imaging, well-described in our case allowed undelayed therapy. CONCLUSION: CAPS and HELLP syndrome, both severe microangiopathic disorders, may be associated. Nosological distinction does not modify treatment strategy, which is a maternal and foetal emergency, but their overlapping requires aggressive and early management.
Subject(s)
Antiphospholipid Syndrome/complications , HELLP Syndrome/diagnosis , Infarction/complications , Liver/blood supply , Pregnancy Complications , Adrenal Cortex Hormones/administration & dosage , Adrenal Cortex Hormones/therapeutic use , Adult , Anticoagulants/administration & dosage , Anticoagulants/therapeutic use , Antiphospholipid Syndrome/drug therapy , Aspirin/administration & dosage , Aspirin/therapeutic use , Drug Therapy, Combination , Emergencies , Female , Humans , Infarction/diagnosis , Infarction/diagnostic imaging , Liver/diagnostic imaging , Magnetic Resonance Imaging , Phenindione/administration & dosage , Phenindione/analogs & derivatives , Phenindione/therapeutic use , Platelet Aggregation Inhibitors/administration & dosage , Platelet Aggregation Inhibitors/therapeutic use , Pregnancy , Time Factors , Tomography, X-Ray ComputedABSTRACT
Long-term survival of patients with chronic lymphocytic leukemia (CLL) is over 10 years, and such patients are thus potential kidney recipients in the case of superimposed end-stage renal disease. However, the renal and patient outcome in this condition is unknown. We report the charts of four patients with CLL who were engrafted in France with a deceased-donor kidney and underwent routine triple immunosuppressive therapy. The results show that these patients developed severe infectious episodes (fatal in one case) and tumoral complications including rapid progression of CLL in two cases. Moreover, the graft may be infiltrated and damaged by monoclonal B cells: one patient lost his graft 14 months after transplantation. Various therapeutic options (modifications of the immunosuppressive regimen, anti-CD20 antibodies, irradiation of the graft) showed little (if any) efficacy. Therefore, we believe that CLL is a too hazardous condition to envisage solid organ transplantation with a routine immunosuppressive regimen, and we propose a more appropriate approach.